Fig. 2. Domain structure of glycogenin and the Glg proteins. The yeast Glg proteins are aligned with mammalian glycogenin, exemplified here by the human form (HsGN). Also shown is an alternate form (HsGNs) that is identical up to residue 202 but then has a distinct COOH terminus (section...
Crystal structureTransglucosylationUDP-glucose hydrolysisThe X-ray structure of rabbit glycogenin containing the T82M (T83M according to previous authors amino acid numbering [1]) mutation causing glycogenosis showed the loss of Thr82 hydrogen bond to Asp162, the residue involved in the activation ...
The storage material had, unlike normal glycogen, a partly fibrillar structure by electron microscopy. None of the patients showed signs or symptoms of muscle weakness but a skeletal muscle biopsy in one case revealed muscle fibres with abnormal glycogen storage. Glycogenin-1 deficiency is known as...
Moreover, an original structure was discovered. This is a ring of glycogen surrounding a finger- or pleat-like plasma membrane invagination. This structure could be physiologically significant in terms of channelling glucose to or from glycogen reserves. Keywords: glycogen, yeast, Thiery reaction, ...
Structure-guided mutagenesis perturbing interactions with phosphorylated tails led to increased basal/unstimulated GS activity. We propose that multivalent phosphorylation supports GS autoinhibition through interactions from a dynamic "spike" region, allowing a tuneable rheostat for regulating GS activity. This...
Further studies are required to elucidate the exact role of this cellular structure and how it constitutes, probably through the control of the cellular localization of the enzymes implicated, an additional regulatory mechanism of the glycogen synthesis and degradation processes. Acknowledgements We thank...
and RBCK1 mutations in that PGBM2 muscles harbor bigger round or oval subsarcolemmal or cytoplasmic inclusions sometimes including myonuclei, ultrastructurally characterized by a lobulated grape structure is always present and each acinus is separated by a thin rim of normally structured and electron ...
The cardiomyocyte storage material was characterized by large inclusions of periodic acid and Schiff positive material that was partly resistant to alpha-amylase treatment consistent with polyglucosan. The storage material had, unlike normal glycogen, a partly fibrillar structure by electron microscopy. ...
The first complex [(W2S4)(WS4)2]2- having the central W2S42+ core is generated from acidification of (WS4)2- in anhydrous medium by acetic acid. The structure of the syn isomer has been determined by X-ray crystallography. The syn isomer crystallizes in space group P1, with a = ...
The glycogen granule itself is essentially a tiered structure of glucose units (i.e., polymers) that is formed in a branch-like structure via 1:4 and 1:6 glycosidic bonds. Glycogen granules are formed on the protein glycogenin and can be as large as 42 nm in diameter as well as having...